Vasculitides Flashcards
What are vasculitides
Inflammatory processes in the walls of affected blood vessels
systemic or constitutional Sx w/ evidence of organ dysfunction
How do large vessel vasculitides present
limb claudication
asymetric BP
absence of pulse, bruits, aortic dilation
How do medium vessel vasculitides present
cutaneous nosules ulcers digital gangrene mononeuritis multiplex microaneurysms
How do small vessel vasculitides present
Purpura, vesicobullous lesions, urticaria, splinter hemorrhages
Necrotizing granulomas, uveitis, scleritis
Glomerulonephritis
Alveolar hemorrhage
What is polymyalgia rheumatica
vasculitis associated with GCA
50+
Women mostly
How does polymyalgia rheumatica present
stiffness, aching, pain in muscles of neck, shoulder, pelvic girdle
Difficulty combing hair, putting on coat, rising form chair
Fever, malaise, weight loss
How do you diagnose polymyalgia rheumatica
Clinically (pain and stiffness)
anemia
elevated acute phase reactants
How do you treat polymyalgia rheumatica
Prednisone low dose (10-20) x 2-4 weeks- will respond in 72 hrs!
Slow taper
monitor for GCA development
These markers tell you it is polymyalgia rheumatica and not another vasculitis
NO muscle weakness
Does not cause blindness
Responds to low dose prednisone
What is polyarteritis nodosa
Necrotizing inflammation of medium vessels and small arteries 2/2 PMN infiltration, may be associated with Hep B (NOT genetics)
Where does polyarteritis nodosa typically show up
skin joints muscles peripheral nerves GI kidney (NOT the lungs!)
S/Sx of polyarteritis nodosa are
Extremity arthralgia, myalgia, or neuropathy
Skin ulcers*, distal gangrene
Foot drop (peripheral mononeuritis)
Intestinal angina
Renal insufficiency (but NO glomerulonephritis)
Labs for polyarteritis nodosa will show
anemia, leukocytosis, elevated ESR
ANCA- Negative
(make sure you test for HBsAg and HBeAg)
To diagnose polyarteritis nodosa you need
Tissue biopsy of an involved organ
Angiogram finding aneurysmal dilations in renal, mesenteric, or hepatic arteries
How do you treat polyarteritis nodosa
High dose corticosteroids
Refractory: Cyclophosphamide (prophylaxis against P. jirovecii)
What is the prognosis of polyarteritis nodosa
No Tx: poor
Tx: 90% 5 yr survival w/ rare remissions
Poor prognostic factors in polyarteritis nodosa are
CKD (Cr >1.6) Proteinuria (>1g x day) GI ischemia CNS disease Cardiac involvement
What is Kawasaki disease
mucocutaneous lymph node syndrome affecting mostly boys, < 5 (peak 2 y/o)
Likely infectious origin
Will show fever, skin findings, and cardiac abnormalities
To diagnose Kawasaki disease, you need __ and 4/5 of these findings
Fever (>38.5 x 5 days) PLUS
- Conjunctivitis (b/l, non-exudative)
- Rash (early perineal erythema and desquamation; macular morbilliform or targetoid lesions to trunk/extremities)
- Extremity changes (hand and foot edema)
- Adenopathy (anterior cervical)
- Mucositis (strawberry tongue cracked lips)
Kawasaki disease lab findings include
Leukocytosis w/ left shift Normocytic normochromic anemia elevated acute phase reactants elevated ALT/AST Thrombocytosis Pyuria *Need ECG/Echo to r/o coronary artery aneurysms
How do you treat Kawasaki disease
2g/kg IVIG admin over 10-12 hrs
80-100mg/kg ASA in 4 divided doses
F/u with ECG
What is Granulomatosis Polyangitis (wegener’s granulomatosis)
Vasculitis of small arteries and capillaries affecting mostly Upper/Lower respiratory (necrotizing granulomatous lesions), and Kidney (glomerulonephritis)
40-50 y/o
M=W
If not treated, can be fatal
S/Sx of GPA are
Nasal congestion Sinusitis otitis media mastoiditis gingivitis stridor Migratory oligoarthritis Scleritis/anterior uveitis Purpura/Dysesthesia
Systemic findings in GPA are
Cough
Dyspnea
Hemoptysis
Fever, malaise, weight loss
On PE of GPA you may find
congestion crusting ulceration bleeding perforation of nasal septum UA: proteinuria, RBC cells and casts
What imaging is needed to diagnose GPA
CT! Much better than CXR
GPA labs will show
ANCA antibodies
Anti-neutrophil cytoplasmic antibodies
Autoimmune vasculitis
Need thoracoscopic lung biopsy to diagnose
How do you treat GPA
chemo agent! Cyclophosphamide + Corticosteroids OR Rituximab + corticosteroids PO QD Methotrexate PCP prophylaxis
What is eosinophilic GPA (Churg-Strauss syndrome)
Vasculitis of small and medium arteries, MC involving the lung
Presents with chronic rhinosinusitis, Asthma*, Eosinophilia (15k+ or >10%), peripheral neuropathy, palpable purpura, subcutaneous nodules, and cardiac Sx (HF, dysrhythmia, pericarditis)
Diagnostics for EGPA are
ANCA in 30-60%
Consolidations, nodules, or ground glass on CT
GOLD: lung biopsy!
How do you treat EGPA
Systemic steroids
advanced/refractory: Immunosuppressive (cyclophosphamide)
Vasculitis phase complications of EGPA can lead to
cardiac failure, MI cerebral hemorrhage renal failure GI bleeding status asthmaticus -w/o Tx, 50% mortality w//in 3 months of Sx
What is the MC systemic vasculitis in kids
IgA vasculitis: Henoch-Schoenlein purpura
IgA deposits around vessel walls
Symptoms of Henosh Schoenlein are
acute onset fever
palpable purpura to LE and buttocks
Purpura + Arthritis + glomerulonephritis + Abd pain
*(three big highlights: purpura of LE, arthritis of knee/ankle, kidneys hematuria)
Labs for Henoch Schoenlein show
elevated IgA Direct immunofluorescence (not sure what it shows)
How do you treat Henoch Schoenlein
Self limited usually, so pt ed, support, analgesics, low dose steroids
We are mainly worried about the HTN
What is Behcet’s syndrome
Vasculitis affecting A&V of all sizes
Highest in mediterranean, middle eastern, and east asians (silk road disease)
20-40 y/o
Sporadic familial clustering
How does Behcet’s present
Recurrent, painful oral ulcers (may be urogenital)
Erythema nodosum- like or Folicular rash
Anterior or posterior uveitis
How do you treat Behcet’s disease
Topical/intralesional steroids for aphthae
Prednisone
Other meds you can try (not as pref): colchicine, MTX, Thalidomide, cyclosporine, TNA-a inhibitors
